Evaluation of management of desmoids tumours associated with familial adenomatous polyposis in Dutch patients
نویسندگان
چکیده
منابع مشابه
Familial Adenomatous Polyposis-Associated Desmoids Display Significantly More Genetic Changes than Sporadic Desmoids
Desmoid tumours (also called deep or aggressive fibromatoses) are potentially life-threatening fibromatous lesions. Hereditary desmoid tumours arise in individuals affected by either familial adenomatous polyposis (FAP) or hereditary desmoid disease (HDD) carrying germline mutations in APC. Most sporadic desmoids carry somatic mutations in CTNNB1. Previous studies identified losses on 5q and 6q...
متن کاملDesmoid Tumours in Familial Adenomatous Polyposis
The term desmoid was first used by Muller [1] to describe the tendon-like aspect and the hard consistence of this type of proliferation (desmos in Greek means band). Desmoid tumours (DTs) are classified as extraor intraabdominal. The extra-abdominal DTs arise from fascial or muscoloaponeurotic structures predominantly of the abdominal wall (Fig 1a, 1b, 1c) and occasionally of the shoulder girdl...
متن کاملDesmoid tumours in familial adenomatous polyposis
Desmoids are rare, benign fibromatous lesions, which can arise in patients with familial adenomatous polyposis (FAP), a disorder caused by germline adenomatous polyposis coli (APC) gene mutation. This study investigated the risk of desmoids in FAP, the relation between specific APC gene mutations and desmoid formation, and the clinical characteristics ofFAP patients with desmoids. Eighty three ...
متن کاملFamilial Adenomatous Polyposis-Associated Papillary Thyroid Cancer.
Papillary thyroid carcinoma (PTC) associated with familial adenomatous polyposis (FAP) is rare. It is usually associated with the cribriform-morular variant of PTC, with unusual patterns on detailed histology examination. This variant is known to have a good prognosis. Papillary thyroid carcinoma associated with FAP commonly occurs in females in their 30s and rarely in the elderly. We report a ...
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ژورنال
عنوان ژورنال: British Journal of Cancer
سال: 2011
ISSN: 0007-0920,1532-1827
DOI: 10.1038/bjc.2011.50